Ventriculocoronary Artery Connections With the Hypoplastic Left Heart: A 4-year Prospective Study: Incidence, Echocardiographic and Clinical Features
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Ventriculocoronary connections (VCCs), also called sinusoids, occur with hypoplastic left heart (HLH). Previous reports are limited to case reports, pathologic series, and surgical series with limited detail, which may underestimate the incidence and overestimate the severity of VCCs in HLH. A study was conducted to determine the incidence VCCs in HLH, their effect on survival, and their echocardiographic and clinical features. The echocardiograms and medical records of 100 consecutive neonatal HLH cases were analyzed. All had an aortic and a mitral valve diameter and a left ventricular (LV) volume less than Z-3. For palliation, Norwood, Sano, or hybrid procedures were used, and if the patient was alive, subsequent bidirectional Glenn and extracardiac Fontan procedures were applied. Cases were classified as manifesting mitral and aortic atresia (MAAA), mitral and aortic stenosis (MSAS), or mitral stenosis and aortic atresia (MSAA). All other diagnoses or any case with additional cardiac anomalies were excluded from the study. Overall, VCCs were found in 15% of the cases. They occurred in 56% of the MSAA subtype cases and were not statistically associated with a high mortality rate. However, in one case, large and multiple VCCs definitely caused or contributed to early death. All VCCs had a transmyocardial course, a turbulent color-Doppler flow, and a dominant usually retrograde systolic coronary artery flow pattern. The VCCs were associated (p < 0.05) with MSAA, endocardial fibroelastosis, and ascending aortic size less than 2 mm. As shown by the findings, 15% of the HLH patients had MSAA with VCCs. Unless the VCCs were large or extensive, they did not contribute to mortality. Detailed echocardiographic analysis of VCCs in HLH was feasible. Recent reports emphasize more severe cases.
KeywordsHypoplastic left heart syndrome Sinusoids Ventriculocoronary connections
- 7.Bertram H, Hitz M, Ono M, Sasse M, Wessel A, Breymann T, Yelbuz M (2008) Hypoplastic left heart syndrome with left ventricular myocardial sinusoids: echocardiographic and angiographic findings in the first neonate surviving the Norwood I and II procedure. Circulation 117:319–321CrossRefGoogle Scholar
- 13.Lang R, Bierig M, Devereux R, Flachskampf F, Foster E, Pellikka P, Picard M, Roman M, Seward J, Shanewise J, Solomon S, Spencer K, Sutton M, Stewart W (2005) Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a Branch of the European Society of Cardiology. J Am Soc Echocardiogr 12:1440–1463Google Scholar
- 22.Vida VL, Bacha EA, Larrazabal A, Gauvreau K, Thiagaragan R, Fynn-Thompson F, Pigula FA, Mayer JE, del Nido PJ, Tworetzky W, Lock JE, Marshall AC (2007) Hypoplastic left heart with intact or highly restrictive atrial septum: surgical experience from a single center. Ann Thorac Surg 84:581–586CrossRefPubMedGoogle Scholar
- 23.Vida VL, Bacha EA, Larrazabal A, Gauvreau K, Dorfman AL, Marz G, Geva T, Marshal AC, Pigula FA, Mayer JE, del Nido PJ, Flynn-Thomas F (2008) Surgical outcome for patients with mitral stenosis aortic atresia variant of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 135:339–346CrossRefPubMedGoogle Scholar